Παρασκευή 16 Μαρτίου 2018

Screening Tests Reveal High Risk Among Adjudicated Adolescents of Auditory Processing and Language Disorders

Purpose
The study investigated the prevalence of risk factors for auditory processing and language disorders among adolescents residing at a local juvenile detention center.
Method
A total of 782 adjudicated adolescents with normal hearing were screened with the Randomized Dichotic Digits Test (Strouse & Wilson, 1999) and the Dichotic Words Test (Moncrieff, 2015). A subset of 420 of those adolescents was also screened with the Clinical Evaluation of Language Fundamentals (CELF; Semel, Wiig, & Secord, 2003).
Results
More than 70% of the adolescents produced weakness on at least 1 dichotic listening test. One third of those produced weakness across both dichotic listening tests, consistent with a binaural integration deficit pattern. Nearly 48% of the subgroup of adolescents produced CELF scores that fell below the criterion for age. Dichotic listening and language scores were more associated in participants with poor performance in both ears during dichotic tests, who also produced the lowest scores on the CELF. There was no main effect of race, but 17- and 18-year-old Black adolescents produced lower CELF scores than White adolescents of the same age.
Conclusions
School-age children referred for disciplinary action may have undiagnosed deficits in auditory processing and/or language. Efforts to screen, diagnose, and remediate these deficits could lead to improvements in communication, learning, and language skills in this population.

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Screening Tests Reveal High Risk Among Adjudicated Adolescents of Auditory Processing and Language Disorders

Purpose
The study investigated the prevalence of risk factors for auditory processing and language disorders among adolescents residing at a local juvenile detention center.
Method
A total of 782 adjudicated adolescents with normal hearing were screened with the Randomized Dichotic Digits Test (Strouse & Wilson, 1999) and the Dichotic Words Test (Moncrieff, 2015). A subset of 420 of those adolescents was also screened with the Clinical Evaluation of Language Fundamentals (CELF; Semel, Wiig, & Secord, 2003).
Results
More than 70% of the adolescents produced weakness on at least 1 dichotic listening test. One third of those produced weakness across both dichotic listening tests, consistent with a binaural integration deficit pattern. Nearly 48% of the subgroup of adolescents produced CELF scores that fell below the criterion for age. Dichotic listening and language scores were more associated in participants with poor performance in both ears during dichotic tests, who also produced the lowest scores on the CELF. There was no main effect of race, but 17- and 18-year-old Black adolescents produced lower CELF scores than White adolescents of the same age.
Conclusions
School-age children referred for disciplinary action may have undiagnosed deficits in auditory processing and/or language. Efforts to screen, diagnose, and remediate these deficits could lead to improvements in communication, learning, and language skills in this population.

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Screening Tests Reveal High Risk Among Adjudicated Adolescents of Auditory Processing and Language Disorders

Purpose
The study investigated the prevalence of risk factors for auditory processing and language disorders among adolescents residing at a local juvenile detention center.
Method
A total of 782 adjudicated adolescents with normal hearing were screened with the Randomized Dichotic Digits Test (Strouse & Wilson, 1999) and the Dichotic Words Test (Moncrieff, 2015). A subset of 420 of those adolescents was also screened with the Clinical Evaluation of Language Fundamentals (CELF; Semel, Wiig, & Secord, 2003).
Results
More than 70% of the adolescents produced weakness on at least 1 dichotic listening test. One third of those produced weakness across both dichotic listening tests, consistent with a binaural integration deficit pattern. Nearly 48% of the subgroup of adolescents produced CELF scores that fell below the criterion for age. Dichotic listening and language scores were more associated in participants with poor performance in both ears during dichotic tests, who also produced the lowest scores on the CELF. There was no main effect of race, but 17- and 18-year-old Black adolescents produced lower CELF scores than White adolescents of the same age.
Conclusions
School-age children referred for disciplinary action may have undiagnosed deficits in auditory processing and/or language. Efforts to screen, diagnose, and remediate these deficits could lead to improvements in communication, learning, and language skills in this population.

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Phonak Adds to Its Hearing Aid and Speech Understanding Technologies

Nadia B-R RIC.jpgPhonak has introduced two lithium-ion rechargeable hearing aids geared to the needs of specific patient populations—those with severe to profound hearing loss and children. Now in its fifth generation, Phonak’s Naída B, for the first time, offers a rechargeable receiver-in-canal option. Naída B provides the industry’s best speech intelligibility index results for those with severe to profound hearing loss, according to the company. Phonak has also added a rechargeable option to its Sky B products, which are designed specifically for children. Sky B-PR gives pediatric patients a full day of uninterrupted hearing on a single charge. Sky B features AutoSense Sky OS and SoundRecover2, which gives children access to a broader range of sounds essential for speech and language development.
 
The company has also launched its new MultiBeam Technology (MBT), the next generation of the Roger 2.4 GHz wireless technology first introduced in 2013. MBT uses three microphones to form six directional beams within 360 degrees. When a microphone with MBT is placed on a table, it automatically selects the speaker to improve speech understanding in group conversations and noisy situations. Powered by MBT are Phonak’s Roger Select, which is equipped with three modes (automatic, manual, lapel) to enable wireless hearing in different environments, and Roger Table Mic II, which is ideal for working adults who need to actively participate in meetings. Phonak has also added the Roger Repeater, which extends the operating range of any Roger network in the Roger for Education portfolio and is ideal for school applications like auditoriums and gymnasiums.
 
The Phonak Naída B and Sky B are now available in the United States, with the rest of the world to follow this month. All new Roger products will be available later in spring 2018.
Published: 3/16/2018 12:24:00 PM


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Phonak Adds to Its Hearing Aid and Speech Understanding Technologies

Nadia B-R RIC.jpgPhonak has introduced two lithium-ion rechargeable hearing aids geared to the needs of specific patient populations—those with severe to profound hearing loss and children. Now in its fifth generation, Phonak’s Naída B, for the first time, offers a rechargeable receiver-in-canal option. Naída B provides the industry’s best speech intelligibility index results for those with severe to profound hearing loss, according to the company. Phonak has also added a rechargeable option to its Sky B products, which are designed specifically for children. Sky B-PR gives pediatric patients a full day of uninterrupted hearing on a single charge. Sky B features AutoSense Sky OS and SoundRecover2, which gives children access to a broader range of sounds essential for speech and language development.
 
The company has also launched its new MultiBeam Technology (MBT), the next generation of the Roger 2.4 GHz wireless technology first introduced in 2013. MBT uses three microphones to form six directional beams within 360 degrees. When a microphone with MBT is placed on a table, it automatically selects the speaker to improve speech understanding in group conversations and noisy situations. Powered by MBT are Phonak’s Roger Select, which is equipped with three modes (automatic, manual, lapel) to enable wireless hearing in different environments, and Roger Table Mic II, which is ideal for working adults who need to actively participate in meetings. Phonak has also added the Roger Repeater, which extends the operating range of any Roger network in the Roger for Education portfolio and is ideal for school applications like auditoriums and gymnasiums.
 
The Phonak Naída B and Sky B are now available in the United States, with the rest of the world to follow this month. All new Roger products will be available later in spring 2018.
Published: 3/16/2018 12:24:00 PM


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Phonak Adds to Its Hearing Aid and Speech Understanding Technologies

Nadia B-R RIC.jpgPhonak has introduced two lithium-ion rechargeable hearing aids geared to the needs of specific patient populations—those with severe to profound hearing loss and children. Now in its fifth generation, Phonak’s Naída B, for the first time, offers a rechargeable receiver-in-canal option. Naída B provides the industry’s best speech intelligibility index results for those with severe to profound hearing loss, according to the company. Phonak has also added a rechargeable option to its Sky B products, which are designed specifically for children. Sky B-PR gives pediatric patients a full day of uninterrupted hearing on a single charge. Sky B features AutoSense Sky OS and SoundRecover2, which gives children access to a broader range of sounds essential for speech and language development.
 
The company has also launched its new MultiBeam Technology (MBT), the next generation of the Roger 2.4 GHz wireless technology first introduced in 2013. MBT uses three microphones to form six directional beams within 360 degrees. When a microphone with MBT is placed on a table, it automatically selects the speaker to improve speech understanding in group conversations and noisy situations. Powered by MBT are Phonak’s Roger Select, which is equipped with three modes (automatic, manual, lapel) to enable wireless hearing in different environments, and Roger Table Mic II, which is ideal for working adults who need to actively participate in meetings. Phonak has also added the Roger Repeater, which extends the operating range of any Roger network in the Roger for Education portfolio and is ideal for school applications like auditoriums and gymnasiums.
 
The Phonak Naída B and Sky B are now available in the United States, with the rest of the world to follow this month. All new Roger products will be available later in spring 2018.
Published: 3/16/2018 12:24:00 PM


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Nonverbal Visual Sequential Learning in Children With Cochlear Implants: Preliminary Findings

The authors tested the hypothesis that children with cochlear implants (CIs) experience domain-general deficits in sequential learning. Twenty children with CIs and 40 children with normal hearing (NH) participated. Participants completed a serial reaction time task that measured implicit sequential learning. During random sequence phases, the CI group had significantly slower reaction times than the NH group. However, there were no significant differences in the rates of sequential learning between groups. Age at implantation was not significantly associated with learning rate in the CI group. Children with CIs demonstrated nonverbal sequential learning that is comparable to children with NH. Contrary to previous research, early auditory deprivation may not be associated with deficits in domain-general sequential learning, but may affect sequential processing. Further investigation is needed to understand the mechanisms underlying the overall delayed reaction times of children with CIs. ACKNOWLEDGMENTS: This research was supported by the following grants: NIH-NIDCD: P50DC000242 and American Speech-Language-Hearing Foundation Clinical Research Grant. The authors had full editorial control of this study and article. The authors have no conflicts of interest to disclose. Address for correspondence: Kelsey E. Klein, University of Iowa, 250 Hawkins Drive, Iowa City, IA 52242. E-mail: kelsey-e-klein@uiowa.edu Received July 11, 2017; accepted January 17, 2018. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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Nonverbal Visual Sequential Learning in Children With Cochlear Implants: Preliminary Findings

The authors tested the hypothesis that children with cochlear implants (CIs) experience domain-general deficits in sequential learning. Twenty children with CIs and 40 children with normal hearing (NH) participated. Participants completed a serial reaction time task that measured implicit sequential learning. During random sequence phases, the CI group had significantly slower reaction times than the NH group. However, there were no significant differences in the rates of sequential learning between groups. Age at implantation was not significantly associated with learning rate in the CI group. Children with CIs demonstrated nonverbal sequential learning that is comparable to children with NH. Contrary to previous research, early auditory deprivation may not be associated with deficits in domain-general sequential learning, but may affect sequential processing. Further investigation is needed to understand the mechanisms underlying the overall delayed reaction times of children with CIs. ACKNOWLEDGMENTS: This research was supported by the following grants: NIH-NIDCD: P50DC000242 and American Speech-Language-Hearing Foundation Clinical Research Grant. The authors had full editorial control of this study and article. The authors have no conflicts of interest to disclose. Address for correspondence: Kelsey E. Klein, University of Iowa, 250 Hawkins Drive, Iowa City, IA 52242. E-mail: kelsey-e-klein@uiowa.edu Received July 11, 2017; accepted January 17, 2018. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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Nonverbal Visual Sequential Learning in Children With Cochlear Implants: Preliminary Findings

The authors tested the hypothesis that children with cochlear implants (CIs) experience domain-general deficits in sequential learning. Twenty children with CIs and 40 children with normal hearing (NH) participated. Participants completed a serial reaction time task that measured implicit sequential learning. During random sequence phases, the CI group had significantly slower reaction times than the NH group. However, there were no significant differences in the rates of sequential learning between groups. Age at implantation was not significantly associated with learning rate in the CI group. Children with CIs demonstrated nonverbal sequential learning that is comparable to children with NH. Contrary to previous research, early auditory deprivation may not be associated with deficits in domain-general sequential learning, but may affect sequential processing. Further investigation is needed to understand the mechanisms underlying the overall delayed reaction times of children with CIs. ACKNOWLEDGMENTS: This research was supported by the following grants: NIH-NIDCD: P50DC000242 and American Speech-Language-Hearing Foundation Clinical Research Grant. The authors had full editorial control of this study and article. The authors have no conflicts of interest to disclose. Address for correspondence: Kelsey E. Klein, University of Iowa, 250 Hawkins Drive, Iowa City, IA 52242. E-mail: kelsey-e-klein@uiowa.edu Received July 11, 2017; accepted January 17, 2018. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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Keratinocyte Growth Factor (KGF) Modulates Epidermal Progenitor Cell Kinetics through Activation of p63 in Middle Ear Cholesteatoma

Abstract

The basal stem/progenitor cell maintains homeostasis of the epidermis. Progressive disturbance of this homeostasis has been implicated as a possible cause in the pathogenesis of epithelial disease, such as middle ear cholesteatoma. In many cases of stem/progenitor cell regulation, the importance of extracellular signals provided by the surrounding cells is well-recognized. Keratinocyte growth factor (KGF) is a mesenchymal-cell-derived paracrine growth factor that specifically participates in skin homeostasis; however, the overexpression of KGF induces middle ear cholesteatoma. In this study, two kinds of thymidine analogs were transferred at different time points and we investigated the effects of overexpressed KGF on the cell kinetics of stem/progenitor cells in vivo. As a result, BrdU(+)EdU(+) cells (stem/progenitor cells) were detected in the thickened epithelium of KGF-transfected specimens. The use of a high-resolution microscope enabled us to analyze the phosphorylated level of p63 in individual nuclei, and the results clearly demonstrated that BrdU(+)EdU(+) cells are regarded as progenitor cells. In the overexpression of KGF, the stimulation of progenitor cell proliferation was inhibited by SU5402, an inhibitor for tyrosine kinase of KGFR. These findings indicate that KGF overexpression may increase stem/progenitor cell proliferation and block terminal differentiation, resulting in epithelial hyperplasia, which is typical in middle ear cholesteatoma.



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Introducing Short Interpulse Intervals in High-Rate Pulse Trains Enhances Binaural Timing Sensitivity in Electric Hearing

Abstract

Common envelope-based stimulation strategies for cochlear implants (CIs) use relatively high carrier rates in order to properly encode the speech envelope. For such rates, CI listeners show poor sensitivity to interaural time differences (ITDs), which are important for horizontal-plane sound localization and spatial unmasking of speech. Based on the findings from previous studies, we predicted that ITD sensitivity can be enhanced by including pulses with short interpulse intervals (SIPIs), to a 1000-pulses-per-second (pps) reference pulse train. We measured the sensitivity of eight bilateral CI listeners to ITD while systematically varying both the rate at which SIPIs are introduced (“SIPI rate”) and the time interval between the two pulses forming a SIPI (“SIPI fraction”). Results showed largely enhanced ITD sensitivity relative to the reference condition, with the size of the improvement increasing with decreasing SIPI rate and decreasing SIPI fraction. For the lowest SIPI fraction, insertion of extra pulses brought ITD sensitivity to the level measured for low-rate pulse trains with rates matching the SIPI rates. The results appear promising for the goal of enhancing ITD sensitivity with envelope-based CI strategies by inserting SIPI pulses at strategic times in speech stimuli.



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Successful preimplantation genetic diagnosis by targeted next-generation sequencing on an ion torrent personal genome machine platform.

Successful preimplantation genetic diagnosis by targeted next-generation sequencing on an ion torrent personal genome machine platform.

Oncol Lett. 2018 Apr;15(4):4296-4302

Authors: Hao Y, Chen D, Zhang Z, Zhou P, Cao Y, Wei Z, Xu X, Chen B, Zou W, Lv M, Ji D, He X

Abstract
Hearing loss may place a heavy burden on the patient and patient's family. Given the high incidence of hearing loss among newborns and the huge cost of treatment and care (including cochlear implantation), prenatal diagnosis is strongly recommended. Termination of the fetus may be considered as an extreme outcome to the discovery of a potential deaf fetus, and therefore preimplantation genetic diagnosis has become an important option for avoiding the birth of affected children without facing the risk of abortion following prenatal diagnosis. In one case, a couple had a 7-year-old daughter affected by non-syndromic sensorineural hearing loss. The affected fetus carried a causative compound heterozygous mutation c.919-2 A>G (IVS7-2 A>G) and c.1707+5 G>A (IVS15+5 G>A) of the solute carrier family 26 member 4 gene inherited from maternal and paternal sides, respectively. The present study applied multiple displacement amplification for whole genome amplification of biopsied trophectoderm cells and next-generation sequencing (NGS)-based single nucleotide polymorphism haplotyping on an Ion Torrent Personal Genome Machine. One unaffected embryo was transferred in a frozen-thawed embryo transfer cycle and the patient was impregnated. To conclude, to the best of our knowledge, this may be the first report of NGS-based preimplantation genetic diagnosis (PGD) for non-syndromic hearing loss caused by a compound heterozygous mutation using an Ion Torrent Personal Genome Machine. NGS provides unprecedented high-throughput, highly parallel and base-pair resolution data for genetic analysis. The method meets the requirements of medium-sized diagnostics laboratories. With decreased costs compared with previous techniques (such as Sanger sequencing), this technique may have potential widespread clinical application in PGD of other types of monogenic disease.

PMID: 29541197 [PubMed]



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MECOM-associated syndrome: a heterogeneous inherited bone marrow failure syndrome with amegakaryocytic thrombocytopenia.

MECOM-associated syndrome: a heterogeneous inherited bone marrow failure syndrome with amegakaryocytic thrombocytopenia.

Blood Adv. 2018 Mar 27;2(6):586-596

Authors: Germeshausen M, Ancliff P, Estrada J, Metzler M, Ponstingl E, Rütschle H, Schwabe D, Scott RH, Unal S, Wawer A, Zeller B, Ballmaier M

Abstract
Heterozygous mutations in MECOM (MDS1 and EVI1 complex locus) have been reported to be causative of a rare association of congenital amegakaryocytic thrombocytopenia and radioulnar synostosis. Here we report on 12 patients with congenital hypomegakaryocytic thrombocytopenia caused by MECOM mutations (including 10 novel mutations). The mutations affected different functional domains of the EVI1 protein. The spectrum of phenotypes was much broader than initially reported for the first 3 patients; we found familial as well as sporadic cases, and the clinical spectrum ranged from isolated radioulnar synostosis with no or mild hematological involvement to severe bone marrow failure without obvious skeletal abnormality. The clinical picture included radioulnar synostosis, bone marrow failure, clinodactyly, cardiac and renal malformations, B-cell deficiency, and presenile hearing loss. No single clinical manifestation was detected in all patients affected by MECOM mutations. Radioulnar synostosis and B-cell deficiency were observed only in patients with mutations affecting a short region in the C-terminal zinc finger domain of EVI1. We propose the term MECOM-associated syndrome for this heterogeneous hereditary disease and inclusion of MECOM sequencing in the diagnostic workup of congenital bone marrow failure.

PMID: 29540340 [PubMed - in process]



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